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Pediatric WAGR Patient with Aniridia-associated Glaucoma: A Case Report
Patricia Abigail Lim-Tanjutco1, Maria Imelda R Yap-Veloso1,2,3
1Rizal Medical Center, Pasig City, Philippines.
Insights
WAGR syndrome, a rare condition, can cause severe glaucoma in children. Surgical intervention like trabeculectomy-trabeculotomy is crucial for managing intraocular pressure and preserving vision.
Area of Science:
- Ophthalmology
- Genetics
- Pediatric Medicine
Background:
- WAGR syndrome is a rare genetic disorder associated with ocular malformations, notably aniridia.
- Glaucoma is a frequent and severe complication in WAGR syndrome, leading to a high risk of vision loss.
- Managing pediatric glaucoma in rare syndromes presents unique challenges, especially in resource-limited environments.
Abstract:
WAGR syndrome is a rare congenital disorder, occurring in approximately 1 in 500,000 to 1,000,000 individuals, often presenting with ocular malformations such as aniridia. Glaucoma frequently develops when the iris and angle structures are affected, posing a significant risk of vision loss. We report a one-year and seven-month-old patient who presented with corneal opacity of the left eye. Examination revealed corneal opacity, aniridia, and markedly elevated intraocular pressure of 65 mmHg, while the fellow eye, also with aniridia, was normotensive. The patient underwent immediate combined trabeculectomy-trabeculotomy. Postoperative follow-up and timely management of complications allowed acceptable pressure control over one year, though visual prognosis remained guarded. This case highlights the challenges of managing glaucoma in WAGR syndrome, particularly in resource-limited settings. Medical therapy alone is often insufficient, making surgical intervention essential. Combined trabeculectomy-trabeculotomy proved effective in maintaining pressure control when glaucoma drainage devices were not feasible. Multiple interventions and close monitoring are frequently required due to the risk of scarring and postoperative complications. Our experience emphasizes the need for a multidisciplinary ophthalmology approach to optimize outcomes. Despite pressure control, visual outcomes often remain poor due to structural anomalies and the challenges inherent to pediatric patients with this rare syndrome.
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